A 79 year old man with a recent episode of non-sustained ventricular tachycardia was admitted to his local hospital for evaluation. ECG demonstrated antero-lateral T-wave inversion (Figure 1). He initially declined to undergo coronary angiogram and was referred for a CMR study as a non-invasive test to assess the presence of inducible ischemia and myocardial viability to guide further management. Previous echocardiography demonstrated mild concentric left ventricular hypertrophy with normal left ventricular (LV) function.

Adenosine stress perfusion demonstrated two distinct patterns of abnormality (Figure 3 andMovie 3): A localised subendocardial perfusion defect in the mid and apical lateral wall was seen, along with a distinct but separate circumferential subendocardial and mid wall defect in the apical hypertrophied regions. Two distinct patterns of scarring/fibrosis were also seen on late gadolinium enhanced (LGE) images: a focal subendocardial infarction in the mid to apical lateral wall and patchy mid wall fibrosis in the apical hypertrophied segments.

Figure 3 Movie 3

Conclusion:

These two patterns of perfusion abnormalities and LGE suggested dual pathologies. The localised increase in apical wall thickening with associated microvascular dysfunction and mid-wall fibrosis are all hallmarks of apical hypertrophic cardiomyopathy (HCM), whereas the localised infarction of the mid and distal left circumflex (LCx) territory with mild peri-infarct perfusion defect are features of ischemic heart disease (Figure 3, lower row).

The patient subsequently underwent a coronary angiogram. This demonstrated moderate diffuse disease in the left anterior descending and right coronary arteries, and a significant lesion of the mid-distal LCx (Figure 4). Based on the vessel anatomy and on the mild ischemic burden, the patient was managed medically.

Figure 4

Perspective:

In this case, CMR was key in establishing the final diagnosis of dual pathologies. Apical HCM was a new finding and indeed of importance with regard to prognosis and family screening. The role of CMR to diagnose apical HCM "missed" by echocardiography has been well described and the detection of fibrosis and microvascular ischaemia may be of benefit for risk stratification and prognosis.1,2,3 Furthermore, the role of CMR in assessing burden of ischemia with stress perfusion and viability in ischemic heart disease is also well described. 4,5

In this patient, apical hypertrophy with associated microvascular dysfunction and patchy mid-wall LGE are typical of HCM. The subendocardial infarction with peri-infarct ischemia in the LCx territory confirms ischemic heart disease. However, it remains unclear whether in this case the arrhythmic substrate for the episode of non-sustained ventricular tachycardia was related to fibrosis or ischaemia related to apical HCM or coronary artery disease. Both mechanisms have been described in triggering arrhythmias in ischemic and non-ischemic cardiomyopathies.3,6,7

Have your say: What do you think? Latest posts on this topic from the forum

Re: Number 11-04: One Question with Two Answers
Re t1 mapping.T1 mapping alone i think will be of limited use. T1 measurement used to calculate volume of distribution (which correlates with collagen volume fraction - see Flett Circ 2010) is clearly more interesting. The fuzzy grey...
On: 03/10/2011 By: drflett Read more?

Re: Number 11-04: One question with two answers
Good point Roshan. Did the patient undergo an angio already? At the Chest he/she must have ... On: 03/09/2011 By: chiarabd Read more?

Re: Number 11-04: One question with two answers
Great case.We scanned a patient last week with previous CABG who had recurrence of chest pain. He had asymmetrical anterior and anteroseptal hypertrophy (15mm+). The perfusion study showed both subendocardial and separate areas of diffuse patchy perfusion defects. The... On: 03/07/2011 By: roshanpw Read more?

Re: Number 11-04: One question with two answers
Where does T1 mapping stand in the discussion of high-res LGE? Could quantifying diffuse fibrosis in some type of HCM also help? Would that be a more traceable prognostic or treatment strategy in these cases? ... On: 03/02/2011 By: jfernandes4125 Read more?

Re: Number 11-04: One question with two answers
So here are a few questios with my thoughts: how many HCM patients in your experience have vasodilator stress induced perfusion defects?- I would say in my experience, >65% have evidence of inducible perfusion defects. Now what to... On: 02/28/2011 By: rohanlon Read more?

Re: Number 11-04: One question with two answers
Hey Rory, see previous post.You have done adenosine stress perfusion scanning in 100 or so HCM patients.Let me set you up here.a) how many HCM patients in your experience have vasodilator stress induced perfusion defectsb)... On: 02/28/2011 By: moon Read more?

Re: Number 11-04: One question with two answers
Very intriguing case. In this COTW the diagnosis of apical HCM is clear with SSFP sequences before the adenosine stress, whereas the finding of iducible ischemia is an "unexpected" finding. We know from the literature that ischemia occurs in... On: 02/28/2011 By: montilor Read more?